Table of Content

15 June 2015 Volume 33 Issue 6

  

The progress of podocyte autophagy and PI3K/Akt signaling pathway 

YU Li, GE Lin

. 2015, 33(6):  501.  doi:10.3969j.issn.1000-3606.2015.06.001

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Autophagy is the process that cells degradate the damaged organelles and the macromolecules by lysosomes. It is a phenomenon of life which is widely existed in nature and plays an important role in maintaining the stability of cellular structure. Current studies showed that the PI3K/Akt signaling pathway, which involved in multiple physiological and pathological processes, is one of the important signaling pathways of autophagy. Recent studies found that the levels of podocyte autophagy and the podocyte diseases have close relationship with PI3K/Akt signaling pathway. In this article, the relationship between autophagy and the PI3K/Akt pathway will be reviewed.


New understanding of complement in childhood immune and/or inflammatory renal diseases

XIAO Huijie, XU Ke, DING Jie

. 2015, 33(6):  504.  doi:10.3969 j.issn.1000-3606.2015.06.002

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The complement system, the chief component of innate immunity, is not only required for host defense against pathogens and homeostasis, but also related to the pathogenesis and development of various kidney diseases. Recent study has shown that tissue-derived complement and immune cell-derived complement can each mediate local inflammation. The complement system acts as a bridge between innate and adaptive immunity. Furthermore it’s also a functional bridge between pathogenic humoral and cellular immune responses in an array of kidney diseases. Increasing evidence links inappropriate complement activation and deficiencies of complement proteins to the pathogenesis of kidney autoimmune disease, ischemia-reperfusion injury, transplant rejection and complications in hemodialysis. The development of pharmacologic agents that target complement in patients with this assortment of immune and/or inflammatory kidney diseases has the potential to abrogate disease progression and improve patient health.

Analysis of clinical pathology of the IgA nephropathy and purpura nephritis in children from the perspective of IgA vasculitis

DUAN Cuirong,XIAO Huijie, ZHONG Xuhui, WANG Fang, GUAN Na, LIU Xiaoyu, DING Jie

. 2015, 33(6):  511.  doi:10.3969 j.issn.1000-3606.2015.06.003

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Objectives To analysis clinical pathology of organ specific IgA vasculitis (IgA nephropathy) and systemic IgA vasculitis (allergic purpura) of purpura nephritis in children. Methods Clinical and pathological data of hospitalized pediatric patients of IgA nephropathy and purpura nephritis were retrospectively analyzed from June 1993 to November 2014. Results There were 405 patients of IgA nephropathy (256 males and 149 females). The ratio of male to female was 1.7:1. The average age was 10.2±2.8 years. The nephrotic syndrome (31.6%) was the most common clinical type, followed by hematuria and proteinuria (27.9%). There were 548 patients of purpura nephritis, 329 males and 219 females. The ratio of male to female was 1.5:1. The average age was 10.2±3.1 years. The hematuria and proteinuria (61.6%) was the most common clinical type, followed by nephrotic syndrome (21.4%). None of the IgA nephropathy progressed to systemic vasculitis (allergic purpura). Conclusions The causes, onset ages and clinical manifestations of IgA nephropathy and allergic purpura may be consistent or overlap, but none of IgA nephropathy (organ specificity IgA vasculitis) progressed to allergic purpura (systemic IgA vasculitis). IgA nephropathy might have more renal immune disorder mechanisms involved in its pathogenesis.

Clinical and pathological features of Henoch-Schönlein purpura nephritis in children

LIU Rui, MA Lu, LI Changchun

. 2015, 33(6):  515.  doi:10.3969 j.issn.1000-3606.2015.06.004

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Objective To investigate the clinical and pathological features of Henoch-Schönlein purpura nephritis (HSPN) in children. Methods The clinical and pathological data of 242 children with HSPN admitted from January 2009 to January 2014 were retrospectively analyzed. Results The most common clinical type was proteinuria and hematuria (135 cases, 55.79%). The mainly pathological types were type Ⅱ and Ⅲ. The distribution of pathological types was statistically significant between different clinical types (P=0.000). The patients with normal urine test had the lowest pathological grade. The patients with isolated hematuria and proteinuria mainly had pathological type Ⅱ. The patients with hematuria and proteinuria mainly had pathological type Ⅲa. The patients with nephrotic syndrome mainly had pathological type Ⅲb. The length of the courses at renal biopsy was not statistically significant among different clinical types (P>0.05) and pathological types (P>0.05). The deposition of immune complex in kidney was not statistically significant among different pathological types (P>0.05). The levels of IgA, C3, and platelet count were not statistically significant among different clinical and pathological types (P>0.05). Conclusion The clinical classification is related to the pathological grade in children with HSPN.

Clinical significance of determination of the expression levels of TGF-β1 and IL-18 mRNA in children with primary nephrotic syndrome

LIU Tao, ZHANG Bili, Wang Jian

. 2015, 33(6):  520.  doi:10.3969 j.issn.1000-3606.2015.06.005

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Objective To study the clinical significance of transforming growth factor (TGF)-β1 and interleukin (IL)-18 mRNA in the diagnosis and evaluation of disease severity of primary nephrotic syndrome (PNS) in children. Methods The expression levels of TGF-β1 and IL-18 mRNA in peripheral blood mononuclear cells (PBMC) were measured by real-time fluorescent quantitative polymerase reaction (RT-PCR) in 77 children with PNS and 30 healthy children (control group). Results In children with steroid-sensitive nephrotic syndrome (SSNS), the expression levels of TGF-β1 and IL-18 mRNA before treatment and at 1, 4 weeks after hormone therapy were significantly lower in children with simple type nephrotic syndrome than those in children with nephritic type nephrotic syndrome (P<0.05). The level of TGF-β1 mRNA before treatment was significantly lower in children with SSNS than that in children with steroid-resistant nephrotic syndrome (SRNS). With the extension of hormone therapy, the expression levels of TGF-β1 mRNA showed a trend of declining in children with PNA. In children with SRNS, the expression levels of TGF-β1 and IL-18 mRNA before treatment and at 1, 4 weeks after hormone therapy were significantly lower in children with simple type NS than those in children with nephritic type nephrotic syndrome (P<0.05). In children with SSNS, the levels of TGF-β1 and IL-18 mRNA were significantly higher in acute phase than those in the remission phase. Conclusions At the early stage of PNS, the detection of the expression levels of TGF-β1 and IL-18 mRNA is useful for evaluatation of disease activity, clinical identification of nephritic type nephrotic syndrome and simple type nephrotic syndrome and early prediction of SRNS.

The changes of pathogens distribution and antimicrobial resistance in children with urinary tract infection in 10 years

CHEN Ye, XIE Yongqiang, WEI Qing, LI Yingjie, GAO Yan

. 2015, 33(6):  525.  doi:10.3969 j.issn.1000-3606.2015.06.006

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Objective To investigate the changes of pathogens distribution and antimicrobial resistance in children with urinary tract infection (UTI) in 10 years. Methods The results of urine culture and drug sensitivity in children with UTI from January 2001 to December 2003, and from January 2011 to December 2013 were retrospectively analyzed．Results In recent 10 years, there was no obvious change in the ratio of gram-negative bacteria to gram-positive bacteria. Escherichia coli was still the main bacteria causing UTI in children. The detection rate of enterococcus was significantly increased from 18.3% in 2011-2013 to 7.5% in 2001-2003 (P<0.05) and it had become the second pathogenic bacteria. The isolation rate of ESBLs producing strains was significantly higher in 2011-2013 than in 2001-2003 (P<0.05). The rate of Escherichia coli sensitive to imipenem remained at 100% and it is also sensitive to enzyme inhibitors antibiotics and nitrofuranto. Sensitivities to antibiotics were changed in different species of enterococcus. Conclusions The distribution of pathogens and antimicrobial resistance in children with UTI are constantly changing. The clinician should pay close attention to changes of epidemiology in the region and hospital and rational use of antimicrobial drugs.

Steroid-resistant nephrotic syndrome accompanied with irreversible leukoencephalopathy: one case report

WANG Jianjun, WU Chun, LIU Zengrong, WANG Si, YANG Jie, LU Xiaojiang, QIN Li

. 2015, 33(6):  528.  doi:10.3969 j.issn.1000-3606.2015.06.007

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Objectives To analyze the diagnosis and management of steroid-resistant nephrotic syndrome (SRNS) accompanied with irreversible leukoencephalopathy in children. Methods The clinical, laboratory and imaging data were retrospectively analyzed in a SRNS child accompanied with irreversible leukoencephalopathy. A literature review was performed. Results After clinical diagnosis of SRNS, glucocorticoid, immunosuppressant, and hemodialysis were administrated for 10 months. During the course of treatment, the seizures, visual problems, and hypertension were repeatedly occured. The cranial MRI showed bilateral occipital parietal lobe hyperintensity and right frontotemporal lobe hyperintensity on T2-weighted imaging and bilateral occipital parietal lobe hypointensity on T2-Flair imaging, which indicated that encephalomalacia was accompanied with gliosis. Conclusions A variety of reasons may induce leukoencephalopathy in children. The accompanied irreversible leukoencephalopathy should be strongly considered in management of SRNS.

Lowe syndrome with novel OCRL mutations in Chinese children: report of two cases

JI Lina, CHEN Chaoying, LI Huarong,DU Peiwei

. 2015, 33(6):  531.  doi:10.3969 j.issn.1000-3606.2015.06.008

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Objective To study the clinical and genetic features of Lowe syndrome. Methods The clinical data and test results of OCRL gene from two children with Lowe syndrome were analyzed. The related literatures were reviewed. Results Two male patients all presented with low molecular proteinuria, hypercalciuria, rickets and nephrolithiasis. Patient 2 had renal tubular acidosis, glycosuria and cryptochism. Patient 1 was found to have abnormal vision and congenital cataract soon after birth and treated surgically. Patient 1 also had psychomotor retardation and the cranial magnetic resonance imaging (MRI) showed agenesis of the corpus callosum. Patient 2 did not have obviously extra-renal symptoms, but was found to have mild cataract by a meticulous ophthalmological examination. MRI showed cerebral hypoplasia and myelination delay and mental retardation was gradually appeared during follow-up. Two OCRL gene mutations were detected. A splice site mutation NG_008638.1:g.46846-46848delTAA/insC was found in patient 1 and a frame shift mutation NM_000276.3:c.321delC in exon 5 was found in patient 2. Both mutations were not reported previously. Conclusions The diagnosis of Lowe syndrome is mainly by clinical manifestations and test of OCRL gene. Lowe syndrome needs to be included in the differential diagnosis of a patient with congenital cataract and renal tubulopathy. Two novel mutations in the OCRL gene were identified.

Changes of surfactant protein-D in serum and cerebrospinal fluid in children with viral encephalitis

WANG Wenjuan,WANG Xueyu

. 2015, 33(6):  535.  doi:10.3969 j.issn.1000-3606.2015.06.009

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Objective To investigate the changes of the pulmonary surfactant protein-D (SP-D) in serum and cerebrospinal fluid in children with viral encephalitis (VE). Methods The levels of SP-D in serum and cerebrospinal fluid were detected by a double-antibody sandwich enzyme-linked immunosorbent assay and compared in thirty children with VE in acute and convalescent phases and in 12 children without VE. Results The levels of SP-D in serum and cerebrospinal fluid between groups of VE acute phase and convalescent phase and no VE were statistically significant (F=103.58，118.15, all P<0.01). The levels of SP-D in serum and cerebrospinal fluid in children with VE in acute phase and in convalescent phase were significantly lower than children without VE (P<0.01). The levels of SP-D in serum and cerebrospinal fluid in children with VE in convalescent phase were all significantly higher than those in acute phase (P<0.01). In children with VE, the level of SP-D in cerebrospinal fluid was weakly correlated negatively with the count of nucleated cells. Conclusions SP-D might be involved in the pathogenesis in VE. The detection of SP-D in serum and cerebrospinal fluid has a certain value for diagnosis of VE.

The serum levels of creatine kinase, brain natriuretic peptide, and neuropeptide Y in severe pneumonia pediatric patients combined with heart failure

ZHANG Linghuan, YU Yanhui, ZHU Xiaoping

. 2015, 33(6):  539.  doi:10.3969 j.issn.1000-3606.2015.06.010

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Objective To investigate the levels of creatine kinase (CK-MB), brain natriuretic peptide (BNP), and neuropeptide Y (NPY) in severe pneumonia pediatric patients combined with heart failure (HF). Methods Pneumonia pediatric patients admitted from December 2010 to December 2014 had been enrolled and divided into pneumonia group (P) (n=32), severe pneumonia group (SP) (n=20) and severe pneumonia combined HF group (HF) (n=36). Twenty healthy children served as control group (C). Serum level of CK-MB was detected by enzyme rate method and were measured by ELISA. BNP and NPY were measured again during the recovery period in 18 cases in HF group. Results The serum levels of CK-MB, BNP and NPY were significantly different among the four groups, (F=25.19 ~ 277.94, P <0.01). The serum levels of CK-MB, BNP and NPY were significantly higher in HF group than in SP, P and C group (P<0.05). The serum level of CK-MB was significantly higher in SP group than that in P and C group (P <0.05) and there was no difference between P and C group (P>0.05). The serum levels of BNP and NPY were not statistically significant between SP and P and C group (all P> 0.05). In HF group, the serum levels of CK-MB, BNP, and NPY were significantly decreased in 18 cases after treatment. The serum level of BNP was positively correlated with CK-MB and NPY (r = 0.681, 0.525, all P<0.01) and the NPY and CK-MB levels were also positively correlated (r=0.545, P <0.01). Conclusions The Detection of the serum levels of CK-MB, BNP and NPY can help diagnose severe pneumonia combined with HF. The BNP maybe the most sensitive indicator.

The variation of insulin like growth factor-I and glucose and correlation in children with sepsis

TIAN Yang, ZHU Cuiping, HONG Jie, HUANG Yiling, MAI Jianning

. 2015, 33(6):  543.  doi:10.3969 j.issn.1000-3606.2015.06.011

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Objective To explore the variation of insulin like growth factor-I (IGF-I) and glucose and correlation in children with sepsis. Methods Forty-two children with sepsis in pediatric intense care unit were enrolled from January 2009 to January 2010. In the morning (2nd morning) after admission, the blood glucose, serum IGF-I, cortisol, insulin, IL-6, and IGF-binding protein-I (IGFBP-1) were detected. In the 3rd and 5th morning, the serum IGF-1 was detected again. According to the blood glucose level of the 2nd morning, the children with sepsis were divided into hyperglycemia group and normal group. Meanwhile, 60 healthy children were served as control group. The data had been compared among three groups. Results In the 2nd morning, the levels of blood glucose, serum IGF-I, cortisol, insulin, and IL-6 were significantly different among three groups (all P<0.05), but the serum IGFBP-I was not significantly different (P>0.05). Compared with control group, the sepsis children with hyperglycemia and with normal blood glucose all had significantly higher serum levels of cortisol and IL-6, and significantly lower serum level of IGF-I. In the 2nd, 3rd, and 5th morning, the serum levels of IGF-1 were not significantly changed with time in sepsis children with hyperglycemia and with normal blood glucose (all P>0.05). Meanwhile, there were no significant differences in the serum levels of IGF-1 between sepsis children with hyperglycemia and with normal blood glucose in the 2nd, 3rd, and 5th morning (all P>0.05). In children with sepsis, the blood glucose and serum IGF-1 was not correlated in the next morning (r=0.152, P=0.267). Conclusions The serum level of IGF-I decreased but maintain stable in children with sepsis. The change of blood glucose may be not related with IGF-I.

Risk and protective factors of periventricular-intraventricular hemorrhage in preterm infants

XU Yinquan, DONG Ya, LIN Zhenlang

. 2015, 33(6):  548.  doi:10.3969 j.issn.1000-3606.2015.06.012

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Objective To identify risk and protective factors of the periventricular-intraventricular hemorrhage (PV-IVH) in preterm infants. Methods By 1:1 case-control study, prenatal and perinatal data were collected and analyzed between preterm infants with PV-IVH and control group from January 2012 to October 2014. The risk and protective factors for the PV-IVH were identified by univariate analysis and multivariate conditional logistic regression analysis. Results There were one hundred and thirty-two preterm infants diagnosed of PV-IVH, in which, among whom 6 preterm infants could not be matched to the control infants in the protocol. Finally, 126 pairs of infants were enrolled in the study. There were no differences between two groups in gestational age and birth weight (all P>0.05). Multivariate conditional logistic regression analysis found that BE<-5 mmol/L in the initial blood gas analysis after birth (OR=1.986, 95.0% CI :1.039-3.796), mechanical ventilation (OR=2.913, 95% CI :1.390- 6.101), weight gain ≤10 g/d in the second week (OR=2.303, 95% CI :1.164-4.558) were risk factors, while number of previous pregnancies ≥1 times (OR=0.426, 95% CI : 0.229-0.792) was a protective factor for PV-IVH. Conclusions The risk factors of PV-IVH in preterm infants include the lower BE value in the initial blood gas analysis, required mechanical ventilation, and less weight gain in the second week.

Monitoring and analysis of birth defects in 73498 infants

YANG Min, WANG Jimei, QIAN Beiqian, DAI Jiale,ZHUANG Xiaolei, CHEN Aiju, MENG Yongqin

. 2015, 33(6):  553.  doi:10.3969 j.issn.1000-3606.2015.06.013

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Objective To understand the occurrence and the related risk factors of birth defects. Methods Descriptive analysis was conducted on birth detect surveillance in the infants during January 2008 to June 2014. Results A total of 777 cases of birth defect were detected in 73498 infants, and the incidence of birth defect was 1.06%. The 5 most common birth defects were congenital heart disease, multi finger (toe), hypospadias, cleft lip, and palate and deformity of external ear. Compared infants born with no birth defects, male, preterm, low birth weight, twin and multiple births and resident were statistically higher in infants with birth defects (P<0.05). The major risk factors of birth defects were the medication history, spontaneous abortion, gestational diabetes mellitus, and family history. Conclusions The incidence of birth defect can be reduced by providing good health care during pre-marriage and pregnant so as to decrease the occurrence of premature infants, twins and multiple births, and low birth weight as well as improving prenatal diagnosis and intensifying birth defects surveillance.

Clinical characteristics and treatment of infective endocarditis in children

LUO Lijuan, CAO Qing, ZHOU Yunfang,PAN Shuhua, WANG Xihua

. 2015, 33(6):  558.  doi:10.3969 j.issn.1000-3606.2015.06.014

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Objective To study the clinical characteristics, treatment and prognosis of infective endocarditis in children. Methords Clinical data from 83 patients of infective endocarditis admitted from 1998 to 2012 were retrospectively analyzed. Results In a total of 83 patients, there were 53 males and 30 females, and the average age was 6.8±4.6 years. The main clinical characteristics were fever (77.1%) and mild to moderate anemia (71.1%). The C-reaction protein (67.5%), erythrocyte sedimentation rate (60.2%), and white blood cell (47.0%) were elevated. Twenty (24.1%) patients had embolism. Blood culture was positive in 56 (67.5%) cases with bacteria mainly being Gram-positive and Streptococcus and Staphylococcus accounted for 89.3%. Vancomycin and other sensitive antibiotics were effective. Neoplasm was detected in 68 cases (82%) by transthoracic echocardiograerphy. Fifty-five (66.2%) patients underwent cardio surgery. Seven patients (8.4%) died. Conclusion In recent years, the distribution of pathogenic bacteria in infective endocarditis had changed. Streptococcus mitis and Staphylococcus aureus has become a major pathogens and need to be treated by vancomycin and other sensitive antibiotics. The detection rate of neoplasm is higher by echocardiography.

A report of four cases of hemolytic disease caused by red blood cell immune in neonates antibodies

XU Wenhao,XIANG Dong

. 2015, 33(6):  562.  doi:10.3969 j.issn.1000-3606.2015.06.015

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Objective To investigate the laboratory findings, clinical manifestations and treatment in hemolytic disease caused by red cell immune antibodies in neonates. Methods The laboratory and clinical data from 4 cases of hemolytic disease of neonates caused by red cell immune antibodies were retrospectively analyzed. Results IgG antibody were detected in all mothers of 4 cases during pregnancy and they were anti-E, anti-D, anti-Jkb and the autoantibody with the titer being 16, 2048, 1 and 16 respectively. The four neonates were all full-term. The jaundice appeared 6 h to 3 d after birth with varying degrees of skin stained yellow, with or without anemia. Serology and elution test found the existence of antibody same as the one on their maternal red cells and the titer was 4, 512, 0, and 2, respectively. All neonates were treated by phototherapy. Two servere cases were also treated by whole blood exchange and red blood cells transfusion. The prognosis were good in all neonates. Conclusions Prenatal immune hematological tests facilitated early detection of irregular erythrocyte antibodies and thus assessment of the risk of hemolytic disease of the fetus and neonates.

Clinical and prognostic analysis of 27 pediatric patients with bronchiectasis caused by Mycoplasma pneumonia and Chlamydia pneumonia

ZHANG Li, ZHOU Tao, FU Simao, LIU Yuling

. 2015, 33(6):  567.  doi:10.3969 j.issn.1000-3606.2015.06.016

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Objective To explore the clinical characteristics, treatment and prognosis of pediatric patients with bronchiectasis caused by Mycoplasma pneumonia (MP) and Chlamydia pneumonia (CP). Methods The clinical data from 27 MP and CP pneumonia pediatric patients with bronchiectasis suggested by the high resolution CT were retrospectively analyzed. Results The morbility rate of bronchiectasis caused by MP and CP pneumonia is 0.56%. The mean age of these patients was 75.4 ± 52.7 months. Among them, 27 cases (100%) had cough, 19 cases (70.4%) had fever, 10 cases (37%) had respiratory distress and 20 cases had lung auscultation. Sixteen cases were MP-IgM positive, 5 cases (18.5%) were CP-IgM positive and 6 cases (22.2%) were positive of both. Eight cases were combined with other pathogens infections, in which 6 cases were bacterial infections. The imaging findings showed diffuse bronchiectasis in 13 cases (48.1%) and local bronchiectasis in14 cases (51.9%). The bronchoscopy found endothelium inflammation, mucosal swelling, partial erosion and follicular hyperplasia in 16 cases (66.7%), the formation of short column sputum bolt in 5 cases (20.8%), in which 1 case had plastic bronchitis. All patients were treated with macrolides antibiotics, 10 cases (37%) combined with methylprednisolone, 3 cases (11.1%) combined with immunoglobulin and 20 cases (74%) combined with other antibiotics. The average length of hospitalization was 12±4.3 days. The bronchiectasis sign disappeared within 4 months in 23 cases (92%). Two cases (8%) still had bronchiectasis after 9 to 15 months, with the recurrent pneumonia. Conclusions MP and CP pneumonia can lead to acute bronchiectasis. Most of patients are recoverable with effective treatment.

Expression of SOX10 in cerebral white matter in immature rats with postnatal infections

SUN Tingting, XU Yan,WANG Jun, WANG Ruiyan, YU Qiao, FENG Jingjing, LI Huihui

. 2015, 33(6):  571.  doi:10.3969 j.issn.1000-3606.2015.06.018

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Objective To explore the effect of postnatal infection on SOX10 expression in cerebral white matter in immature rats. Methods A total of 96 newborn SD rats were randomly divided into hypoxia group, lipopolysaccharide (LPS) group, and control group. At day 3 and 6 after birth, the rats in LPS group and hypoxia group were intraperitoneally injected with 0.25 mg/kg of LPS while the rats in control group were injected with normal saline. Meanwhile the rats in hypoxia group were maintained in a hypoxic tank under atmospheric pressure and thermostatic water bath at 37℃ for 2 hours of ventilation with mixed gas containing 8% O2 and 92% N2 at a rate of 2 L/min starting 3 days after birth. At day 7, 10, 14, 21 after birth, eight rats in each groups were sacrificed and the cerebral white matter was extracted. HE staining was performed to observe the pathological changes of cerebral white matter by light microscopy. The expression of SOX10 in cerebral white matter was determined by immunohistochemical and Western blotting analysis. The expression of TLR-4 was determined by Western blotting. Results In LPS group and hypoxia group, the SOX10 positive cells and expressions of SOX 10 and TLR-4 were increased at day 7, reached the peak at day 10, and then gradually declined. There were significant differences between any two time points (P<0.05). In control group, there were a few positive cells and limited expressions of SOX 10 and TLR-4 and there were no differences between any two time points (P>0.05). At each time point, the difference in the SOX10 positive cells and the expressions of SOX 10 and TLR-4 were statistically significant among three groups (P<0.05) in the order of hypoxia group > LPS group > control group and there were significantly differences between each groups (P<0.05). Conclusions Postnatal infections can lead to cerebral white matter lesions in immature rats. The existence of both hypoxia and infection can aggravate the brain injury. The high expression of SOX 10 may have the protective effect.

Kozlowski type spondylometaphyseal dysplasia: one case report

SUO Lei, LUAN Zuo

. 2015, 33(6):  576.  doi:10.3969 j.issn.1000-3606.2015.06.019

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Objective To investigate the clinical characteristics and diagnostics of Kozlowski type spondylometaphyseal dysplasia (SMDK). Methods The clinical features, laboratory tests and genetic testing of one SMDK case were analyzed. Results A eight-year-old male patient had more than 6 years course of disease. The clinical manifestations were stubby limbs, fingers and toes, varus deformity, knee valgus deformity, scoliosis and lordosis and severe metaphyseal changes. The heterozygous mutations were detected in TRPV4 and NXX3-2 genes. Conclusions Typical clinical features combined with genetic diagnosis facilitate early detection and accurate diagnosis of SMDK.

Cryopyrin-associated periodic syndrome: one case report

ZHAO Peiwei, DING Yan, YIN Wei, YUE Xin, HE Xuelian

. 2015, 33(6):  579.  doi:10.3969 j.issn.1000-3606.2015.06.020

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Objective To investigate the clinical features and genetic basis of cryopyrin-associated periodic syndrome (CAPS). Methods The clinical manifestations, laboratory tests, and genetic tests of one case of CAPS were retrospectively analyzed. The related literatures were reviewed. Results A 7 year and eight month old male patient had recurrent fever for 7 years and his whole body was covered with patchy red rash which was itchy and faded with pressure. The limbs and joints were normal. The levels of high-sensitivity C-reactive protein, erythrocyte sedimentation rate, rheumatoid factors were increased. The patient had fundus arteriosclerosis, double conjunctival lesions and nerve deafness on both sides. There was no mutation found in NLRP3 gene coding region, but a heterozygous mutation (-2667G>T) had been found in 5 ' untranslated region. Compared with normal control, the mRNA level of NLRP3 increased 4.2 times and the expressions of IL-1β and IL-18 gene increased 2.2 (P=0.002) and 1.2 times (P>0.05). Conclusions The clinical features of CAPS can be recurrent fever, rash, and joint involved. The ophthalmologic abnormalities and varying degrees of deafness may occur during the progression. The test of NLRP3 gene may help diagnosis.

Clinical analysis of eosinophilic gastroenteritis in two children and review of literature

ZHU Bizhen, BAI Haitao, WU Jinzhun, XU Jinping

. 2015, 33(6):  583.  doi:10.3969 j.issn.1000-3606.2015.06.021

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Objective To explore the clinical-pathological characteristics, treatment and prognosis of eosinophilic gastroenteritis in children. Methods The data of clinical manifestation, laboratory examination, endoscopy, biopsy, management and prognosis from 2 pediatric patients with eosinophilic gastroenteritis were retrospectively analyzed. Related articles were reviewed. Results The age of two patients was 13 and 14 years old. Case 1 was male and had an onset with acute pancreatitis. Case 2 was female with a history of food allergy and had an onset with unexplained ascites. Both patients presented with significantly increased peripheral eosinophil count (45.9%-64.8%) and serum IgE (246-393 IU/ml). Bone marrow cytology showed increased proportion of eosinophils in both patients. Gastroscopy was performed in case 1 while gastroscopy and colonoscopy endoscopy were performed in Case 2 and the results indicated eosinophilic infiltration in duodenum. Duodenal mucosa biopsy showed eosinophilic infiltration in case 1 while gastrointestinal pathology biopsy showed multiple mucosal eosinophil infiltration in case 2. After the therapy of food avoidance, steroid and anti-allergic drugs, both patients had complete remission 1 week later. Meanwhile the peripheral eosinophil count was decreased to normal. Case 1 was followed up for 24 months, and case 2 was followed up for 2 months. Both patients showed no evidence of relapse. Conclusions The clinical manifestation and endoscopic examination of eosinophilic gastroenteritis in children are protean and lack of specificity. Unexplained gastrointestinal symptoms accompanied with eosinophilia may suggest the possibility of eosinophilic gastroenteritis. Eosinophilic infiltration in ascites and gastrointestinal mucosa strongly indicate the diagnosis of eosinophilic gastroenteritis. After exclusion of other possible diseases, the definite diagnosis can be made.

The clinical application of continuous positive airway pressure in severe bronchiolitis

SHI Ruihe

. 2015, 33(6):  588.  doi:10.3969 j.issn.1000-3606.2015.06.022

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Bronchiolitis is the most common lower respiratory tract disease in infants. There is no specific treatment for it and the treatment is mainly supportive. The management of bronchiolitis is the clinical hotspot recently. Continuous positive airway pressure (CPAP) has gained more attention in clinical application as it is a safe, effective, and noninvasive method and can effectively relieve symptoms of bronchiolitis and improve prognosis. This paper reviews the clinical application of CPAP in infants with severe bronchiolitis.

Current research status of non-antibiotic antibacterial therapies

LONG Zhi

. 2015, 33(6):  592.  doi:10.3969 j.issn.1000-3606.2015.06.023

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With the inappropriate use of antibiotics, the situation of bacterial resistance is more and more severe. The emergence of multidrug-resistant (MDR) bacteria has made it difficult to cure the infections in clinical. For treatment the infections caused by MDR and reducucton of the generation of resistant bacteria, researchers are actively studying on the nonantibiotic substances for antibacterial activity. In this paper, the advances in those with confirmed effects such as phage therapy, metal/chelation therapy, immunization therapy, photodynamic therapy, and nitric oxide (NO)-based therapies, small molecule inhibitors, antimicrobial peptides and Chinese herb were reviewed.

The pathogenesis of the lower respiratory infection by Mycoplasma pneumoniae and the therapeutic effects of glucocorticoid

CAI Jinlong, QU Shuqiang

. 2015, 33(6):  597.  doi:10.3969 j.issn.1000-3606.2015.06.024

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Mycoplasma pneumonia (MP) is one of common pathogens of community-acquired pneumonia in children. In recent years, the incidence of MP pneumonia (MPP) shows a trend of increasing. The report of severe refractory MPP was more and more. Glucocorticoids has anti-inflammatory, anti-allergic and restraining immune response pharmacological effects and is the most effective anti-inflammatory drug currently. In this paper, pathogenesis of the lower respiratory infection by MP and the therapeutic effects of glucocorticoid were reviewed.